| syndroom van congenitale microgastrie met reductiedefect van extremiteit (aandoening) | | syndroom van congenitale microgastrie met reductiedefect van extremiteit | | syndroom van congenitaal abnormaal kleine maag met reductiedefect van extremiteit
| | Congenital microgastria with limb reduction defect syndrome | | Syndrome with the association of microgastria and limb reduction defect. Most of the 50 cases of congenital microgastria reported in the literature are associated with other multiple congenital anomalies. Isolated congenital microgastria is an extremely rare condition; only three cases have been reported in the literature so far. The clinical manifestations of congenital microgastria depend on the stage at which the embryologic development of the stomach is arrested. The microgastria-limb reduction association is believed to arise as a result of aberrant mesodermal development in the fourth or fifth week of embryonic life. The outcome for most of the reported patients with severe microgastria was either death or extreme malnutrition. |
| | Id | 721880009 | | Status | Primitive |
| DHD Diagnosis thesaurus reference set |
| RIVM authorized national diagnosis thesaurus to ICD10 complex mapping reference set | | Target | Q40.2 | | Term | Overige gespecificeerde congenitale misvormingen van maag |
| Target | Q73.8 | | Term | Overige onderontwikkeling van niet gespecificeerde extremiteit(en) |
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| SNOMED CT to Orphanet simple map | 2538 |
| SNOMED CT to ICD-10 extended map | | Target | Q40.2 | | Rule | TRUE | | Advice | ALWAYS Q40.2 | POSSIBLE REQUIREMENT FOR ADDITIONAL CODE TO FULLY DESCRIBE DISEASE OR CONDITION | | Correlation | SNOMED CT source code to target map code correlation not specified |
| Target | Q73.8 | | Rule | TRUE | | Advice | ALWAYS Q73.8 | | Correlation | SNOMED CT source code to target map code correlation not specified |
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