type-I-interferon-geassocieerde auto-inflammatoire aandoening
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Sterile alpha motif domain containing 9 like-associated autoinflammatory syndrome (disorder)
SAMD9L-associated autoinflammatory syndrome
SAMD9L-SAAD - SAMD9L-associated autoinflammatory disease
Sterile alpha motif domain containing 9 like-associated autoinflammatory syndrome
A rare autoinflammatory syndrome characterized by nodular panniculitis, lipoatrophy, severe early-onset interstitial lung disease, and basal ganglia calcifications. Most patients have progressive isolated B-cell and natural killer cell cytopenias. Respiratory failure was also reported.
Id1365682007
StatusPrimitive
Associated morphologyinflammatoire morfologie
Finding sitestructuur van immuunsysteem
Pathological processafwijkend immuunproces
SNOMED CT to ICD-10 extended map
TargetD89.8
RuleTRUE
AdviceALWAYS D89.8 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified