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Синдром гетеротакісії: невизначене розташування внутрішніх органів (situs ambiguus)

ORPHA:157769· ICD-10 Q89.3· Situs ambiguus

Визначення(English summary)

A rare, genetic, developmental defect during embryogenesis characterized by a partial mirror-image transposition of intra-thoracic and/or intra-abdominal organs across the left-right axis of the body. Intra-organ variations and other malformations, such as ciliary motricity anomalies (e.g. Kartagener syndrome), biliary atresia and cardiac defects, are frequently associated. Left (polysplenia syndrome) or right (asplenia syndrome) isomerism are usually observed.

Успадкування
Multigenic/multifactorial
Вік початку
Antenatal