Autosomal recessive hereditary disorder
Bone injury
Congenital anomaly of skeletal bone
Developmental hereditary disorder
Hereditary disorder of musculoskeletal system
Multiple dislocations with dysplasia
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Lethal Larsen-like syndrome (disorder)
Lethal Larsen-like syndrome
Larsen-like syndrome, lethal type, has characteristics of multiple joint dislocation and respiratory insufficiency due to tracheomalacia and or lung hypoplasia. It has been described in less than ten patients. Transmission is thought to be autosomal recessive. Brain dysplasia has been described in some patients. Genetic factors have not been ruled out.
letaal Larsen-achtig syndroom
Id719409004
StatusPrimitive
Associated morphologyDysplasia
Finding siteBone structure
OccurrenceCongenital
Pathological processPathological developmental process
Associated morphologyDislocation
Finding siteJoint structure of multiple body sites
Clinical courseProgressive
Due toSpontaneous event
DHD Diagnosis thesaurus reference set
ICD-10 complex map reference set
TargetQ74.8
RuleTRUE
AdviceALWAYS Q74.8 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified