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Autoinflammatory syndrome with pyogenic bacterial infection and amylopectinosis (disorder)
Autoinflammatory syndrome with pyogenic bacterial infection and amylopectinosis
A rare genetic mixed autoinflammatory and autoimmune syndrome with characteristics of chronic systemic autoinflammation (presenting as recurrent fever in the neonatal or infantile period) and combined immunodeficiency (manifesting as recurrent viral and invasive bacterial infections). Muscular amylopectinosis may be subclinical or be complicated by myopathy/cardiomyopathy.
auto-inflammatoir syndroom met pyogene bacteriƫle infectie en glycogeenstapelingsziekte type 4
auto-inflammatoir syndroom met infectie door pyogene bacterie en glycogeenstapelingsziekte type 4
Id829973009
StatusPrimitive
Associated morphologyInflammatory morphology
Finding siteStructure of immune system
Pathological processAbnormal immune process
Clinical courseChronic
Pathological processAutoimmune process
ICD-10 complex map reference set
TargetM35.8
RuleTRUE
AdviceALWAYS M35.8 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified