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Miller-Dieker syndrome

ORPHA:531Malformation syndromeAutosomal dominantAntenatal, Infancy, Neonatal

Ассоциированные гены (3)

HIC1
HIC ZBTB transcriptional repressor 1
Candidate gene tested in
OMIM: 603825
YWHAE
tyrosine 3-monooxygenase/tryptophan 5-monooxygenase activation protein epsilon
Role in the phenotype of
OMIM: 605066
PAFAH1B1
platelet activating factor acetylhydrolase 1b regulatory subunit 1
Role in the phenotype of
OMIM: 601545

Фенотипы (18)

Очень частый (80–99%)10
HP:0000177Abnormality of upper lip
HP:0000286Epicanthus
HP:0000348High forehead
HP:0000463Anteverted nares
HP:0001250Seizure
HP:0001339Lissencephaly
HP:0001510Growth delay
HP:0002120Cerebral cortical atrophy
HP:0002353EEG abnormality
HP:0003196Short nose
Частый (30–79%)2
HP:0001561Polyhydramnios
HP:0001626Abnormality of the cardiovascular system
Периодический (5–29%)6
HP:0000112Nephropathy
HP:0000960Sacral dimple
HP:0001251Ataxia
HP:0001539Omphalocele
HP:0002079Hypoplasia of the corpus callosum
HP:0004209Clinodactyly of the 5th finger

Эпидемиология (1)

Point prevalence
Unknown
Worldwide

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы