Kagami–Ogata syndrome

Also known as: Paternal UPD14 syndrome

Imprinting disorder with bell-shaped thorax and polyhydramnios.

Medical specialty: Endocrinology
Subspecialty: Endocrinology / Pediatric endocrinology
Sex & age distribution: Male: affected; Female: affected equally; Children: yes, perinatal
Gene(s) involved: DLK1, MEG3, RTL1

Etiology & mechanism

Paternal UPD14.

Gene(s) involved (3)

DLK1MEG3RTL1

Symptoms & signs (6)

abdominal wall defectsbell-shaped thoraxcoat-hanger ribsdevelopmental delaypolyhydramniosrespiratory failure

SyndromeScope — educational reference. Content is model-generated and not clinically verified; always confirm against OMIM, Orphanet and GeneReviews before clinical use.