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AH-amyloïdose (aandoening)
AH-amyloïdose
zwareketenamyloïdose
'heavy chain' amyloidose
AH amyloidosis
Heavy chain amyloidosis
A rare systemic amyloidosis with characteristics of the aggregation and deposition of amyloid fibrils composed of monoclonal immunoglobulin heavy-chain fragments, usually produced by a plasma cell neoplasm. Amyloid fibrils deposit in various organs, most commonly in the kidneys. It typically affects older patients and clinical presentation includes signs and symptoms of renal dysfunction, sometimes leading to nephrotic syndrome and end stage renal disease. Cardiac, liver and nerve involvement has also been described.
Id1231149005
StatusPrimitive
Associated morphologydepositie van amyloïd
Causative agentImmunoglobulin heavy chain
SNOMED CT to Orphanet simple map442582
SNOMED CT to ICD-10 extended map
TargetE85.9
RuleTRUE
AdviceALWAYS E85.9 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified