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Temple syndrome due to paternal 14q32.2 microdeletion

ORPHA:254525Etiological subtypeAutosomal dominant, Not applicableInfancy, 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 (22)

Очень частый (80–99%)6
HP:0000750Delayed speech and language development
HP:0001252Hypotonia
HP:0001270Motor delay
HP:0001773Short foot
HP:0008897Postnatal growth retardation
HP:0200055Small hand
Частый (30–79%)3
HP:0001319Neonatal hypotonia
HP:0001511Intrauterine growth retardation
HP:0011220Prominent forehead
Периодический (5–29%)13
HP:0000119Abnormality of the genitourinary system
HP:0000347Micrognathia
HP:0000358Posteriorly rotated ears
HP:0000826Precocious puberty
HP:0001249Intellectual disability
HP:0001513Obesity
HP:0001627Abnormal heart morphology
HP:0002714Downturned corners of mouth
HP:0004482Relative macrocephaly
HP:0007010Poor fine motor coordination
HP:0008872Feeding difficulties in infancy
HP:0030084Clinodactyly
HP:0031878Acromicria

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