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congenitaal syndroom van gegeneraliseerde hypercontractiele spierstijfheid (aandoening)
congenitaal syndroom van gegeneraliseerde hypercontractiele spierstijfheid
aangeboren syndroom van gegeneraliseerde hypercontractiele spierstijfheid
Congenital generalized hypercontractile muscle stiffness syndrome
A rare defect of tropomyosin characterized by decreased fetal movements and generalized muscle stiffness at birth. Additional features include joint contractures, short stature, kyphosis, dysmorphic features, temperature dysregulation and variably severe respiratory involvement with hypoxemia. Muscle biopsy shows mild myopathic features.
Id1174000008
StatusPrimitive
Associated morphologymorfologische afwijking
Occurrencecongenitaal
Pathological processproces van pathologische ontwikkeling
SNOMED CT to Orphanet simple map476406
SNOMED CT to ICD-10 extended map
TargetG71.2
RuleTRUE
AdviceALWAYS G71.2 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified