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Temple syndrome due to maternal uniparental disomy of chromosome 14

ORPHA:96184Etiological subtypeAntenatal, Neonatal

Ассоциированные гены (3)

MEG3
maternally expressed 3
Role in the phenotype of
OMIM: 605636
DLK1
delta like non-canonical Notch ligand 1
Role in the phenotype of
OMIM: 176290
RTL1
retrotransposon Gag like 1
Role in the phenotype of
OMIM: 611896

Фенотипы (35)

Очень частый (80–99%)6
HP:0000826Precocious puberty
HP:0001252Hypotonia
HP:0001270Motor delay
HP:0001773Short foot
HP:0008897Postnatal growth retardation
HP:0200055Small hand
Частый (30–79%)9
HP:0000750Delayed speech and language development
HP:0001249Intellectual disability
HP:0001382Joint hypermobility
HP:0001511Intrauterine growth retardation
HP:0001513Obesity
HP:0001518Small for gestational age
HP:0001622Premature birth
HP:0001956Truncal obesity
HP:0004322Short stature
Периодический (5–29%)20
HP:0000028Cryptorchidism
HP:0000160Narrow mouth
HP:0000175Cleft palate
HP:0000193Bifid uvula
HP:0000218High palate
HP:0000293Full cheeks
HP:0000322Short philtrum
HP:0000347Micrognathia
HP:0000403Recurrent otitis media
HP:0000445Wide nose
HP:0000463Anteverted nares
HP:0002021Pyloric stenosis
HP:0002650Scoliosis
HP:0003124Hypercholesterolemia
HP:0004904Maturity-onset diabetes of the young
HP:0005280Depressed nasal bridge
HP:0007010Poor fine motor coordination
HP:0011220Prominent forehead
HP:0011968Feeding difficulties
HP:0030084Clinodactyly

Эпидемиология (2)

Cases/families
Worldwide
Point prevalence
<1 / 1 000 000
Worldwide

Лекарства и терапия

Источник: Open Targets Platform (в реальном времени)

Клинические исследования

Источник: ClinicalTrials.gov (в реальном времени)

Внешние ресурсы