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alfathalassemie major (aandoening)
alfathalassemie major
Hb Bart-syndroom
hydrops foetalis van Bart
Hemoglobin Bart's hydrops syndrome
Hemoglobin Barts hydrops
Hemoglobin Bart's disease
Hemoglobin Bart hydrops syndrome
Alpha thalassemia major
A severe form of alpha-thalassemia that is mostly lethal and associated with severe long-term outcome and lifelong transfusions in survivors. It is characterized by fetal onset of generalized edema, pleural and pericardial effusions, and severe hypochromic anemia.
Id5300004
StatusPrimitive
Associated morphologyhydrops
Occurrencefoetale periode
Finding siteerytrocyt
Occurrencecongenitaal
SNOMED CT to Orphanet simple map163596
SNOMED CT to ICD-10 extended map
TargetD56.0
RuleTRUE
AdviceALWAYS D56.0 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION
CorrelationSNOMED CT source code to target map code correlation not specified