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ernstige X-gebonden verstandelijke beperking type Gustavson (aandoening)
ernstige X-gebonden verstandelijke beperking type Gustavson
ernstige X-gebonden mentale retardatie type Gustavson
ernstige X-gebonden verstandelijke handicap type Gustavson
Severe X-linked intellectual disability Gustavson type
This syndrome has characteristics of X-linked intellectual disability, microcephaly, optical atrophy with impaired vision or blindness, a severe hearing defect, facial dysmorphology, spasticity, epileptic seizures and restricted joint movement. It has been described in seven children from two generations of a Swedish family. All patients died in early childhood.
Id722213009
StatusPrimitive
Associated morphologyatrophia
Finding sitestructuur van nervus opticus
Occurrencecongenitaal
Pathological processproces van pathologische ontwikkeling
Associated morphologyafwijkend klein
Finding sitestructuur van hoofd
Occurrencecongenitaal
Pathological processproces van pathologische ontwikkeling
Has interpretationgestoord
Interpretsgehoorfunctie
Has interpretationgestoord
Interpretsintellectueel vermogen
Has interpretationgestoord
InterpretsAdaptation behavior
DHD Diagnosis thesaurus reference set
RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set
TargetF72.9
TermErnstige zwakzinnigheid; Zonder vermelding van gedragsstoornissen
SNOMED CT to Orphanet simple map3078
SNOMED CT to ICD-10 extended map
TargetF72.9
RuleTRUE
AdviceALWAYS F72.9 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified