Chronic mental disorder	Chronic nervous system disorder	Congenital neurological disorder	Degenerative disorder	Developmental hereditary disorder	Hereditary disorder of nervous system	Intellectual disability	Multiple malformation syndrome with facial defects as major feature	X-linked recessive hereditary disease
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X-linked intellectual disability with hypogammaglobulinemia and progressive neurological deterioration syndrome (disorder)
	X-linked intellectual disability with hypogammaglobulinemia and progressive neurological deterioration syndrome
	This syndrome is characterized by moderate intellectual deficit, bilateral single palmar creases, seizures, variable hypogammaglobulinemia and characteristic features (synophrys, prognathism, and hirsutism). It has been reported in three males from two generations of one family. All underwent progressive neurological deterioration.This syndrome is transmitted as an X-linked trait, and the causative gene is located between Xq21.33 and Xq23.
	X-gebonden syndroom van verstandelijke beperking, hypogammaglobulinemie en progressieve neurologische uitval
	X-gebonden syndroom van verstandelijke handicap, hypogammaglobulinemie en progressieve neurologische uitval X-gebonden syndroom van mentale retardatie, hypogammaglobulinemie en progressieve neurologische uitval

Id	719156006
Status	Primitive

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

Clinical course

Associated morphology	Degenerative abnormality
Finding site	Structure of nervous system

DHD Diagnosis thesaurus reference set

ICD-10 complex map reference set

Target	Q87.8
Rule	TRUE
Advice	ALWAYS Q87.8 \| POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
Correlation	SNOMED CT source code to target map code correlation not specified