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THOC6-related developmental delay-microcephaly-facial dysmorphism syndrome

ORPHA:363444Malformation syndromeAutosomal recessiveInfancy, Neonatal

Ассоциированные гены (1)

THOC6
THO complex subunit 6
Disease-causing germline mutation(s) (loss of function) in
OMIM: 615403

Фенотипы (44)

Очень частый (80–99%)8
HP:0000164Abnormality of the dentition
HP:0000348High forehead
HP:0000490Deeply set eye
HP:0000750Delayed speech and language development
HP:0001999Abnormal facial shape
HP:0003189Long nose
HP:0009765Low hanging columella
HP:0040196Mild microcephaly
Частый (30–79%)12
HP:0000010Recurrent urinary tract infections
HP:0000119Abnormality of the genitourinary system
HP:0000278Retrognathia
HP:0000286Epicanthus
HP:0000319Smooth philtrum
HP:0000670Carious teeth
HP:0001263Global developmental delay
HP:0001627Abnormal heart morphology
HP:0002119Ventriculomegaly
HP:0006989Dysplastic corpus callosum
HP:0010864Intellectual disability, severe
HP:0012443Abnormality of brain morphology
Периодический (5–29%)24
HP:0000047Hypospadias
HP:0000054Micropenis
HP:0000077Abnormality of the kidney
HP:0000085Horseshoe kidney
HP:0000122Unilateral renal agenesis
HP:0000215Thick upper lip vermilion
HP:0000220Velopharyngeal insufficiency
HP:0000307Pointed chin
HP:0000337Broad forehead
HP:0000365Hearing impairment
HP:0000689Dental malocclusion
HP:0001328Specific learning disability
HP:0001631Atrial septal defect
HP:0001643Patent ductus arteriosus
HP:0001845Overlapping toe
HP:0002023Anal atresia
HP:0008209Premature ovarian insufficiency
HP:0009890High anterior hairline
HP:0010282Thin lower lip vermilion
HP:0011623Muscular ventricular septal defect
HP:0011682Perimembranous ventricular septal defect
HP:0012382Left-to-right shunt
HP:0012385Camptodactyly
HP:0030127Endometriosis

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